THE SURGICAL IMPLICATIONS OF CHRONIC GRANULOMATOUS-DISEASE
THE SURGICAL IMPLICATIONS OF CHRONIC GRANULOMATOUS-DISEASE
复制标题
DOI：
10.1016/s0002-9610(99)80167-6
复制
发表时间：
1995-03-01
影响因子：
3
通讯作者：
BRANDT, ML
中科院分区：
文献类型：
--
作者：
ECKERT, JW;ABRAMSON, SL;BRANDT, ML
背景：儿童慢性肉芽肿病（CGD）是一种罕见的先天性吞噬细胞NADPH氧化酶系统异常。受影响的中性粒细胞和巨噬细胞有无效的呼吸爆发，不能破坏某些被吞噬的细菌和真菌。CGD患者通常表现为复发性化脓性和真菌性感染。过氧化氢酶阳性细菌经常参与其中，因为它们代谢它们产生的过氧化氢，使其无法用于增强CGD中性粒细胞的杀微生物活性。受折磨的患者也有形成肉芽肿的倾向，这可能导致胃肠道和泌尿生殖道阻塞。方法：回顾10例CGD患者的诊断年龄、手术方式、手术并发症和药物治疗情况。结果：10例患儿中男性8例。首次发病的平均年龄为18个月（2天至9.8岁），每个儿童平均发生9.9次感染，平均每年发生1.4次感染。所有患者均需接受外科手术，平均每人2.9例。5名儿童在诊断为CGD之前因感染接受手术治疗。最常见的手术是软组织脓肿切开引流（7例）或直肠周围脓肿（3例）、胸腔穿刺（3例）和支气管镜检查（3例）。3例患儿术后伤口愈合不良。2例出现部分胃出口梗阻，经抗生素治疗痊愈。一例出现肉芽肿性膀胱炎伴梗阻，对抗生素有反应。结论：由于未确诊的CGD患者可能存在手术问题，外科医生需要熟悉这种情况。对于有复发性或不寻常感染或不明原因的伤口愈合问题的儿童，应怀疑诊断。
BACKGROUND: Chronic granulomatous disease (CGD) of childhood is a rare congenital abnormality of the phagocyte NADPH oxidase system. Affected neutrophils and macrophages have an ineffective respiratory burst and cannot destroy certain phagocytized bacteria and fungi. CGD patients usually present with recurrent pyogenic and fungal infections. Catalase-positive bacteria are frequently involved, since they metabolize the hydrogen peroxide they produce, making it unavailable for augmentation of microbicidal activity in CGD neutrophils. Afflicted patients also have a tendency to form granulomas, which can lead to obstruction of the gastrointestinal and genitourinary tracts.METHODS: Charts of 10 patients with CGD were reviewed for age at diagnosis, surgical procedures, complications of these procedures, and medical treatment.RESULTS: Eight of the 10 children were male. The average age at first presentation was 18 months (range 2 days to 9.8 years), Each child developed a mean of 9.9 infections and an average of 1.4 infections per year. All required surgical procedures, with an average of 2.9 procedures each. Five children had operative procedures for infections that preceded the diagnosis of CGD. The procedures performed most frequently were incision and drainage of soft-tissue abscesses (7) or perirectal abscess (3), thoracentesis (3) and bronchoscopy (3). Three children had poor wound healing following surgery. Two developed partial gastric outlet obstruction which resolved with antibiotic therapy. One developed granulomatous cystitis with obstruction which responded to antibiotics.CONCLUSIONS: Since patients with undiagnosed CGD may present with surgical problems, surgeons need, to be familiar with this condition. The diagnosis should be suspected in children who have recurrent or unusual infections or unexplained problems with wound healing.